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==Medical Therapy== | === Medical Therapy === | ||
*Corticosteroids are commonly used as first line therapy. They are able to produce a rapid decrease in eosinophil counts, which is valuable if there is already evidence of organ dysfunction related to high eosinophil levels. <ref name=":0" /> | |||
**0.5-1mg/kg/day of prednisone (or the equivalent dose of another steroids) is the recommended starting dose. Patients with non-severe disease manifestations can start with lower doses <ref name=":0" /> | |||
**Steroids are tapered over time, with second line agents added if needed to maintain control. | |||
**In one study, corticosteroids were able to induce remission in 85% of patients at 1 month <ref name=":1" /> | |||
*Hydroxyurea is the most commonly used second-line agent for hypereosinophilic syndrome. Typical dose range is 500mg-2g per day. Hydroxyurea acts at the level of the bone marrow to reduce production of new eosinophilis. <ref name=":0" /> As such it is not suitable if a rapid lowering in eosinophil levels is needed. Hydroxyurea is not usually used alone, it is most often used as an additional steroid-sparing agents. | |||
*[[Interferon-alpha]] is also a second-line, steroid sparing agent. Use of this agent is limited by its toxicities. Interferon-alpha also has activity on lymphocytes, so it may be particularly effective in eosinophilia secondary to lymphocytic malignancies. <ref name=":0" /> | |||
*[[Imatinib]] and related TKIs have shown promise in treating [[chronic eosinophilic leukemia]] due to similar molecular re-arrangements as CML. <ref name=":0" /><ref>Pardanani et al. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood 2003 101:3391-3397.</ref> This family of TKIs is very well tolerated. These drugs are most effective in patients who have the FIP1L1-PDGFRA rearrangement driving a clonal expansion in the bone marrow. <ref name=":1" /> | |||
*Anti-IL5 antibody [[mepolizumab]] has shown potential to rapidly reduce eosinophil counts in trials and reduce the need for corticosteroids, however this agent is still investigational. <ref>Rothenberg et al. Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab. NEJM 2008; 358:1215-1228</ref> | |||
*Other cytotoxic agents have been used in isolated cases, but lack strong evidence | |||
*In severe cases refractory to other therapy, stem cell transplant may be considered if the cause of the eosinophilia is a primary bone marrow process. | |||
==References== | ==References== |
Revision as of 21:58, 29 August 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
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Medical Therapy
- Corticosteroids are commonly used as first line therapy. They are able to produce a rapid decrease in eosinophil counts, which is valuable if there is already evidence of organ dysfunction related to high eosinophil levels. [1]
- 0.5-1mg/kg/day of prednisone (or the equivalent dose of another steroids) is the recommended starting dose. Patients with non-severe disease manifestations can start with lower doses [1]
- Steroids are tapered over time, with second line agents added if needed to maintain control.
- In one study, corticosteroids were able to induce remission in 85% of patients at 1 month [2]
- Hydroxyurea is the most commonly used second-line agent for hypereosinophilic syndrome. Typical dose range is 500mg-2g per day. Hydroxyurea acts at the level of the bone marrow to reduce production of new eosinophilis. [1] As such it is not suitable if a rapid lowering in eosinophil levels is needed. Hydroxyurea is not usually used alone, it is most often used as an additional steroid-sparing agents.
- Interferon-alpha is also a second-line, steroid sparing agent. Use of this agent is limited by its toxicities. Interferon-alpha also has activity on lymphocytes, so it may be particularly effective in eosinophilia secondary to lymphocytic malignancies. [1]
- Imatinib and related TKIs have shown promise in treating chronic eosinophilic leukemia due to similar molecular re-arrangements as CML. [1][3] This family of TKIs is very well tolerated. These drugs are most effective in patients who have the FIP1L1-PDGFRA rearrangement driving a clonal expansion in the bone marrow. [2]
- Anti-IL5 antibody mepolizumab has shown potential to rapidly reduce eosinophil counts in trials and reduce the need for corticosteroids, however this agent is still investigational. [4]
- Other cytotoxic agents have been used in isolated cases, but lack strong evidence
- In severe cases refractory to other therapy, stem cell transplant may be considered if the cause of the eosinophilia is a primary bone marrow process.